takayasu arteritis in children and adolescents

Review

Takayasu arteritis in children and adolescents

Juergen Brunner1, Brian M. Feldman2,3, Pascal N. Tyrrell2,Jasmin B. Kuemmerle-Deschner4,5, Lothar B. Zimmerhackl1,Ingmar Gassner5 and Susanne M. Benseler2

Abstract

Takayasu arteritis is a devastating vasculitis of the aorta and its major branches. The clinical manifest-

ations in paediatric patients are less specific than in adults: in children the disease presents with fever,

arthralgias and hypertension. Intramural inflammation results in narrowing of the blood vessel lumen and

therefore hypoperfusion of the parenchyma. Conventional angiography is the gold standard diagnostic

procedure. Corticosteroids, cyclophosphamide, MTX and biological therapies such as TNF-a blocking

agents are treatment options.

Key words: Vasculitis, Angiitis, Children, Takayasu arteritis.

Introduction

Takayasu arteritis (TA) is the most common, granuloma-

tous inflammation of large arteries; it is potentially life

threatening. TA appears to have an acute early phase,

with non-specific symptoms—such as hypertension,

headache, fever, muscle pain, arthralgia, night sweats

and weight loss. Due to the non-specific symptoms and

the absence of specific laboratory parameters, the dis-

ease is often unrecognized in this phase, although the

diagnosis should be made early if possible. If untreated,

during the next phase the disease affects the aorta and its

main branches. Vessel wall inflammation leads to concen-

tric wall thickening, fibrosis and thrombus formation.

Affected vessels may become stenotic or may develop

aneurysms and vascular remodelling. Presenting symp-

toms at this stage commonly reflect end-organ ischae-

mia—such as renal infarction and stroke [1, 2]. Since

large-vessel biopsies are most often not possible, the

diagnosis of TA is based on clinical criteria. Laboratory

investigations should support the diagnosis of TA and

imaging results must be confirmatory. The diagnosis of

TA remains a challenge to clinicians.

To date, few studies on TA in childhood have been pub-

lished [3–5]. This review focuses on the clinical presenta-

tion and diagnosis of TA in childhood and adolescence,

differential diagnosis and therapeutic approach.

Epidemiology

The incidence of TA in adults is estimated to be 2.6/

1 000 000/year in North America and 1/1 000 000/year in

Europe overall [6–8]. The incidence in Sweden is reported

at 1.2/1 000 000, in the UK 0.8/1 000 000 and in Kuwait

2.2/1 000 000 [9–12]. The incidence reported for Japan is

2/1 000 000 [13]. Studies show that the disease is distrib-

uted all over the world and is not restricted to any one

ethnic group. The reported female: male ratio ranges from

1.2 : 1 in Israel to 6.9 : 1 in Mexico [13–15]. The incidence

of TA in children is unknown. Kerr et al. [7] included

30% paediatric patients in their study and reported an

incidence in all ages of 2.6/1 000 000.

Classification and diagnosis

Classification criteria

In 1978, Ishikawa proposed criteria for the clinical diagno-

sis of TA based on an analysis of 96 Japanese patients.

The patients’ age had to be <30 years. Fulfilling the two

major and at minimum one minor criteria or one major and

at least four of nine minor criteria (representing vessel in-

volvement) resulted in a diagnostic sensitivity of 84% and

a specificity of 95% [16]. The patients were only com-

pared with a control group of 12 persons with other

aortic diseases but not with a control group with other

inflammatory vascular diseases [17]. The Ishikawa criteria

RE

VIE

W

1Department of Pediatrics, Medical University Innsbruck, Innsbruck,Austria, 2Division of Rheumatology, Department of Pediatrics, TheHospital for Sick Children, 3Department of Health Policy Managementand Evaluation, Medicine and the Dalla Lana School of Public Health,University of Toronto, Toronto, Canada, 4Department of Pediatrics,Eberhard-Karls University, Tuebingen, Germany and 5Department ofRadiology, Medical University Innsbruck, Innsbruck, Austria.

Correspondence to: Juergen Brunner, Department of Pediatrics,Pediatric Rheumatology, Medical University Innsbruck, Anichstrasse35, 6020 Innsbruck, Austria. E-mail: [email protected]

Submitted 10 March 2010; revised version accepted 28 April 2010.

! The Author 2010. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: [email protected]

RHEUMATOLOGY

Rheumatology 2010;49:1806–1814

doi:10.1093/rheumatology/keq167

Advance Access publication 18 June 2010

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are very detailed and therefore considered somewhat

impractical for clinical practice.

In 1990, the ACR TA classification criteria were pro-

posed, which consisted of: (i) age at disease onset

<40 years; (ii) claudication of the extremities;

(iii) decreased brachial artery pulse; (iv) blood pressure

difference >10 mmHg between arms; (v) bruit over sub-

clavian arteries or aorta; and (vi) arteriogram abnormality,

which is not related to arteriosclerosis or fibromuscular

dysplasia (FMD) [18].

The ACR TA criteria were validated by comparing 63 TA

patients with 744 people suffering from other types of

vasculitis. The addition of laboratory markers including

elevated ESR, anaemia and hypergammaglobulinaemia

improved the test characteristics. Evidence of three or

more criteria led to a specificity of 97.8% and a sensitivity

of 90.5% for TA. A classification tree was constructed with

five of these six criteria, omitting claudication of an ex-

tremity. The classification tree demonstrated a sensitivity

of 92.1% and a specificity of 97.0%. Eighteen TA patients

were children or adolescents [18]. In an effort to optimize

the Ishikawa criteria, Sharma et al. [19] published a

modification in 1995. Their modifications likely made the

Ishikawa criteria even more impractical. In 2007, members

of a consensus conference of the Pediatric Rheumatology

European Society (PRES) suggested consensus criteria

for the classification of childhood vasculitis subtypes

including TA [20, 21]. These were endorsed by the

European League Against Rheumatism. The PRES classi-

fication criteria for childhood TA mandate the evidence of

angiographic abnormalities plus the presence of at least

one of the TA features: (i) decreased peripheral artery

pulse(s) or claudication of extremities; (ii) a blood pressure

difference >10 mmHg; (iii) bruits over the aorta or its major

branches; and (iv) hypertension (related to childhood nor-

mative data) [21]. These criteria have yet to be validated

within cases of childhood vasculitis and TA mimics.

Researchers have attempted to define distinct subtypes

of TA based on angiographic characteristics, which then

could provide a basis for therapeutic decision making

[17, 22–24]. These approaches have not had a great

impact on clinical practice so far.

Clinical features

Clinical diagnosis of TA commonly presents a challenge to

the clinician. It is estimated that one-third of children pre-

sent with inactive, so-called ‘burnt-out’ disease, in which

clinical features represent vascular sequelae rather than

active vasculitis. Both the natural history and the time

from onset of symptoms to diagnosis are variable. It is

likely that the non-specific clinical presentation of child-

hood TA contributes to a delay in diagnosis. The clinical

spectrum at presentation of children with TA differs from

that of adults; however, hypertension is the most common

symptom in both groups. Children frequently have hyper-

tension, headaches, fever and weight loss at diagnosis of

TA [2, 16, 25–54]. The most common presenting features

in adulthood are hypertension and bruits [6, 7, 55–61].

General features

The most frequent presentation in childhood is hyperten-

sion (82.6%), followed by headaches (31%), fever (29%),

dyspnoea (23%), weight loss (22%) and vomiting (20.1%)

[2, 16, 25–54]. Non-specific symptoms such as abdominal

pain (16.6%) and vomiting can herald TA in children [3, 4].

Musculoskeletal symptoms are, overall, observed in only

14% of children with TA [2, 16, 25–54]. However, in South

American TA patients, arthritis appears to be more

common and has been noted in 65% [4]. In contrast,

adult patients rarely report arthritis or arthralgia at diagno-

sis of TA [6, 7, 61, 62]. Clinical data are summarized in

Table 1. TA has been found to be associated with RA and

chronic IBD [59, 63].

Organ-specific features

Organ manifestations result from decreased blood supply

due to vascular stenosis and subsequent ischaemia in the

vascular territory. Claudication (13%) and poor or absent

arterial pulses (13%) occur in areas of vessel involvement

[2, 16, 25–54]. Bruits are uncommonly described.

Secondary cardiac disease is reported in 19% of paedi-

atric patients [2, 16, 25–54]. In contrast, the most common

vascular symptoms in adults are bruits (48%) and claudi-

cation (27%) [6, 7, 55–61]. In paediatric TA, neurological

manifestations include headaches (31%) and stroke (17%)

[2, 16, 25–54]. Skin manifestations including nodules and

rashes are uncommon [4, 64–66]. Skin ulcers are not re-

ported in children. Lymphadenopathy is uncommon [4].

Ocular manifestations such as retinopathy are rare in

children [3, 67, 68].

The diagnosis of childhood TA remains challenging

due to the often non-specific character of symptoms

including headaches, fever, dyspnoea, weight loss, vomit-

ing, abdominal pain and musculoskeletal symptoms. The

combination of systemic symptoms of inflammation,

decreased or absent pulses and possibly intermittent or

activity-related features of organ ischaemia should raise

the level of suspicion for TA. Suspicion of the diagnosis

mandates additional testing.

Common laboratory tests

There is no specific laboratory marker for TA. Classical

inflammatory markers are commonly tested; 53% of chil-

dren had an elevated ESR compared with 45% of adult TA

patients overall. ESR is considered the best available rou-

tine laboratory indicator for disease activity of TA in ado-

lescents [7, 17, 69–71]. However, the ESR may continue to

be elevated in disease remission [7, 69]. CRP is increas-

ingly measured as a disease activity marker in TA. It ap-

pears to correlate well with active TA. High CRP levels

have also been found to be associated with a higher risk

of thrombotic complications in TA [72–74].

Novel laboratory markers

Novel TA markers including tissue plasminogen activator,

intercellular adhesion molecule-1, vascular cell adhesion

molecule-1, E-selectin and platelet endothelial cell adhe-

sion molecule-1 were evaluated in TA and appear to

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TA

BL

E1

Clin

ical

and

lab

ora

tory

chara

cte

ristics

of

paed

iatr

icp

atients

with

TA

Pa

tie

nts

Pa

ed

iatr

icC

ase

se

rie

s

Au

tho

rC

ak

ar

D’S

ou

za

Fie

ldsto

ne

Ha

hn

Ho

ng

Ja

inM

ora

les

Mu

ran

jan

Ca

se

rep

ort

sS

um

ma

ry,

n(%

)A

du

lt,

n(%

)

Year

of

pub

licatio

n2008

1998

2003

1998

1992

2000

1991

2000

1994–2008

Num

ber

of

patients

,n

19

11

631

70

24

26

17

41

241

844

Sex

fem

ale

:male

2.8

:11.2

:17.0

:11.3

:14.3

:15.0

:13.3

:10.8

:12.0

:13.0

:1T

ota

lA

ge

(mean),

years

8–17

(13)

4–17

(9.6

)1.7

–17

(9.9

)2.4

–14.5

(8.4

)3–15

(9.4

)<

18

(14)

3–15

(11.7

)5–11

(9.3

)1–18

(8.5

)1–18

(10)

Lo

catio

n(c

ountr

y)

Turk

ey

Canad

aU

SA

So

uth

Afr

ica

Ko

rea

Ind

iaM

exic

oIn

dia

Dis

trib

ute

d

Genera

l,n

(%)

Fever

5(2

6)

NR

5(8

3)

2(6

)N

R1

(4)

17

(65)

3/(

17)

14

(34)

47/1

60

(29.4

)125

(14.8

)W

eig

ht

loss

2(1

1)

NR

4(6

6)

NR

11

(15)

1(4

)17

(65)

9(2

2)

44/1

99

(22.1

)80

(9.4

)

Chest

pain

NR

2(3

3)

NR

5(7

)2

(11)

6(1

5)

15/1

99

(7.5

)83

(9.8

)

Ab

do

min

alp

ain

7(3

7)

NR

NR

4/(

5)

13

(50)

9(2

2)

33/1

99

(16.6

)6

(0.7

)V

om

itin

gN

R2

(33)

NR

13

(18)

4(1

6)

13

(50)

3/(

17)

5(1

2)

40/1

99

(20.1

)11

(1.3

)

Ep

ista

xis

NR

NR

2(2

)7

(26)

9/1

99

(4.5

)

Palp

itatio

ns

NR

2(3

3)

NR

22

(31)

3(1

2)

2(1

1)

29/1

99

(14.6

)

Co

ug

hN

RN

R11

(15)

2(1

1)

2(5

)15/1

99

(7.5

)H

ead

ache

16

(84)

2(1

8)

NR

29

(41)

13

(54)

3/(

17)

3(7

)66/2

10

(31.4

)212

(25.0

)

Synco

pe

NR

NR

4(1

6)

4/1

99

(2.0

)

Hyp

ert

ensio

n17

(89)

11

(100)

4(6

6)

26

(84)

65

(93)

20

(83)

22

(85)

11

(65)

23

(56)

199/2

41

(82.6

)445

(52.5

)

Derm

alm

anifesta

tio

nN

R3

(50)

2(6

)5

(19)

2(5

)12/2

30

(5.2

)31

(3.7

)A

rthra

lgia

/art

hritis

3(1

6)

NR

2(6

)1

(1)

1(4

)17

(65)

2(1

1)

7(1

7)

33/2

30

(14.3

)143

(16.9

)

Dysp

no

ea

NR

NR

37

(52)

5(2

0)

4(2

3)

3(7

)49/2

10

(23.3

)83

(9.8

)

Org

an

sp

ecific

,n

(%)

Po

or

puls

es

anyw

here

11

(58)

NR

10

(32)

7(4

1)

2(5

)30/2

30

(13.0

)191

(22.5

)

Bru

its

5(2

6)

NR

1(1

6)

12

(38)

14

(58)

6(1

5)

38/2

30

(16.5

)410

(48.4

)

Cla

ud

icatio

n6

(32)

4(3

6)

4(6

6)

4(1

2)

5(7

)8

(30)

1(2

)32/2

41

(13.3

)229

(27.0

)

Str

oke/e

ncep

halo

path

yN

R2

(33)

7(2

2)

15

(21)

4(1

6)

6(3

5)

5(1

2)

39/2

30

(16.9

)64

(7.6

)C

ard

iac

dis

ease

8(4

2)

NR

20

(64)

4(1

6)

8(4

7)

12

(29)

52/2

30

(22.6

)172

(20.3

)

Resp

irato

ryd

isease

2(1

8)

NR

2/2

10

(0.9

)

Uveitis

NR

3(5

0)

2(6

)5

(19)

2(5

)12/2

30

(5.2

)

Lab

ora

tory

,n

(%)

ES

R19

(100)

NR

6(1

00)

23

(83)

37

(52)

10

(42)

19

(73)

11

(65)

16

(39)

141/2

30

(61.0

)383

(45.2

)

The

patients

rep

ort

ed

inclin

ical

stu

die

s[3

,4,

64,

65,

67,

83,

84]

and

case

series

[2,

24,

54]

are

co

mp

are

dw

ith

clin

ical

pre

senta

tio

no

fad

ult

patients

[6,

7,

55–61].

The

perc

enta

ge

of

clin

ical

manifesta

tio

ns

isre

late

dto

the

am

ount

of

paed

iatr

icp

atients

enro

lled

instu

die

saskin

gfo

rth

em

anifesta

tio

n.

NR

:no

tre

po

rted

.

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correlate with other measurements of vascular inflamma-

tion and disease activity [75, 76]. Specific AECA have

been found in patients with TA in recent studies; their

pathogenic role remains unclear [73, 77, 78]. AECA may

cause endothelial cell activation and expression of adhe-

sion molecules, assisting and continuing the inflammation

in TA [79]. Anti-monocyte antibodies and AECA are pre-

sent in a significant proportion of adult patients with TA

and correlate with disease activity [7, 77, 78]. Annexin V is

a regulatory protein in apoptosis. Antibodies directed

against annexin V have been shown to lead to apoptosis

of vascular endothelial cells [80]. Antibodies against

annexin V have been demonstrated in adult patients in

conjunction with AECA [81]. Platelets and pro-coagulatory

markers are frequently increased in TA, possibly causing

a hypercoagulable state and increased risk of thrombus

formation [76, 82].

So far, no TA-specific laboratory marker of disease ac-

tivity is available. Classical inflammatory markers have

limited sensitivity and lack specificity. Raised inflamma-

tory markers are more often than not associated with

active inflammation in TA.

Imaging

A combination of imaging modalities is commonly

required for diagnosis and monitoring of TA in children.

Vascular imaging is accomplished by conventional angi-

ography, magnetic resonance angiography (MRA), CT

angiography (CTA) or Doppler ultrasound. Distinct vessel

wall imaging techniques include gadolinium-enhanced

MRA and more recently fluorodeoxyglucose PET.

The thoracic and abdominal aorta are the vessels most

often involved in childhood TA [3, 4, 64, 65, 67, 83, 84]; the

characteristic appearance on angiography is diffuse aortic

involvement [4]. Recent studies have included 19 patients

investigated with conventional angiography [15] or MRA

[4]. One hundred and thirty-seven vascular lesions were

detected, with stenosis being the most common type of

lesion (53%). Occlusion was present in 21%, dilatation in

16% and aneurysm in 10% of patients. In this series of

studies, the most frequently involved vessels were the

renal arteries (in 73% of the patients), the subclavian

arteries (in 57%) and the carotid arteries (in 52%). The

thoracic or abdominal aorta was affected in �50% of

the children [29]. Several classifications have been pro-

posed based on the distribution of angiographic abnorm-

alities seen in TA [17, 23, 24, 85].

The diagnosis of TA in children is often based on the

findings of conventional angiography of the aorta and its

branches, which is considered the gold standard [22].

Conventional angiography is invasive, is associated with

exposure to a significant radiation dose, requires iodi-

nated contrast material and can be difficult to per-

form—especially in young children or patients with high

degree of vascular stenosis. The strength of the conven-

tional angiogram is the exact visualization of flow in the

blood vessels and the extent of collateralization. It pro-

vides an estimate of ischaemic risk. Conventional angio-

graphy does not provide information about the vessel wall

itself [86–89]. Vascular narrowing may be due to acute

intramural inflammation or chronic wall fibrosis [90],

which are indistinguishable by conventional angiography.

MRA is a less invasive imaging technique. It can provide

calculated, non-dynamic blood flow information. Its

strength is that MRA provides important additional infor-

mation about the vessel wall. Mild inflammation of the wall

without associated significant stenosis will be missed by

conventional angiography but may be picked up by MRA

[87, 88]. Axial T1-weighted MRA best demonstrates ab-

normal wall thickening of the vessels. The inflammatory

oedema of the wall causes a bright T2-weighted signal.

Contrast-enhanced MRA further depicts vessel wall ir-

regularity (Fig. 1). Focal TA disease activity can be deter-

mined in contrast-enhanced MRI, which has been shown

to correlate with clinical and laboratory findings in some

patients [88]. Designated ‘oedema-weighted’ images in

MRI detect fluid, representing inflammation, within the

vessel wall. Evidence of new anatomical lesions with evi-

dence of vessel wall oedema is thought to represent

active disease [91]. Clinical and laboratory features plus

FIG. 1 14 2/12-year-old girl with TA. MRI: sagittal oblique

section: pronounced thickening of the aortic wall in its

entire length Ectasia of the ascending aorta, aortic arch

and descending aorta.


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conventional angiography and MRA represent the most

commonly utilized diagnostic tool set of TA. [91].

CTA provides similar information about disease to MRA

[92]. Spiral CTA with breath-hold technique makes

well-enhanced, thin-section transaxial images available

and reveals luminal narrowing. Additionally, it points out

mural changes as high-attenuation wall changes in pre-

contrast CT images, circumferential wall thickening with

inhomogeneous enhancement or a concentric low-

attenuation ring inside the aortic wall [7, 93]. Maximal

intensity projection images show luminal obstruction or

narrowing or dilatation of the vessels as well. In the

delayed phase mural enhancement is also present

[7, 92, 94]. Radiation exposure has to be considered as

CTA involves a large dose of radiation.

Ultrasonography is useful in establishing an early diag-

nosis of TA [95, 96]. Comparison of ultrasound with angio-

graphy has shown agreement on stenosis in 97% when

examining the common carotids, 95% for the brachio-

cephalic trunks and 97% for the vertebral arteries [97].

Sonography reveals thickening of affected vessel walls,

occlusion and dilatation. Sonography might help to estab-

lish early disease of TA in a pre-stenotic phase in the

extracranial vessels [96]. Alterations in flow velocity can

be determined (Fig. 2A and B). The method is inexpensive

and without radiation exposure. Limitations include the

investigator-dependent quality of the examination.

18FDG-PET is an imaging technique used to assess the

increased glucose metabolism in inflammatory cells.

Some recent studies have concluded that there is a pos-

sible role for 18FDG-PET as a screening method in early

TA, especially for those patients presenting with unchar-

acteristic symptoms [98–100]. It appears that 18FDG-PET

can identify more vascular regions affected by the inflam-

matory process than MRA [101]. However, the PET results

are not specific for vasculitis [102]. Although PET scanning

may provide valuable information about inflammation in

the arterial wall, it cannot show changes in the structure

(and therefore the damage) of the vessel wall nor it can

show luminal blood flow. PET access is limited to large

medical centres; for this reason there is a lack of stand-

ardization of PET imaging for TA.

In conclusion, conventional angiography is still the gold

standard for the diagnosis of TA. MRA and MRI are helpful

in monitoring the disease. Standardized high-resolution

sonography might become an important addition to the

diagnostic armamentarium.

Differential diagnosis

In children with suspected TA, some more common

diagnoses have to be considered. The differential diag-

nosis includes developmental disorders (coarctation and

Marfan syndrome), other autoimmune disorders [pri-

mary vasculitides (Behcet’s disease, Kawasaki disease

and thrombangiitis obliterans), secondary vasculitides

(SLE, SpA and sarcoidosis) or infectious aortitis (tubercu-

losis, syphilis, Staphylococcus aureus, Salmonella,

Treponema, CMV or herpes virus] [103–106]. An associ-

ation of true TA with tuberculosis, in some regions, has

been suggested [104, 107]. Both TA and tuberculosis are

chronic granulomatous diseases. Active tuberculosis has

been recognized in up to 20% of patients with TA [55]. TA

and tuberculosis have been associated with HLA-B al-

leles; however, these have different distributions in TA

and tuberculosis [108]. The role of tuberculosis in the

pathogenesis of TA is therefore unclear. Cross-reactivity

between Mycobacteria and human HSP may play a role

[109]. TA is associated more often with vascular stenosis,

whereas tuberculosis is more often associated with ero-

sion of the vessel wall and aneurysm development. TB

particularly affects the descending thoracic and abdom-

inal aorta [1]. Regarding hypertension, FMD is an import-

ant differential diagnosis [1]. Differentiating TA from FMD

may be a challenge; however, FMD is not an inflammatory

disease.

Therapy

Adequate therapy in TA is important to prevent irreversible

vessel damage with resulting insufficiency of vital organs.

Corticosteroids are still the mainstay of treatment [1].

Other immunosuppressive agents are a therapeutic

option [1].

Glucocorticoids are an effective agent for most patients

with active TA. Remission has been achieved in 60% of

patients treated with glucocorticoids alone [6, 7, 110,

111]. Although improvement of symptoms in TA usually

follows glucocorticoid therapy, relapses usually occur

with dosage reduction [73].

In the most recent survey, 19 children with TA all

received prednisone (2 mg/kg/day). The dose was tapered

after 1–2 months and was thereafter continued at alter-

nate day dosing; the lowest dose was 5–10 mg/alternate

days. Nine patients received additional

FIG. 2 Ultrasound examination of the right carotid artery

in (A) longitudinal and (B) cross-section: marked echo-

genic mural thickening (arrowheads).



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cyclophosphamide and one patient received infliximab.

MTX (10–12.5 mg/m2/week) was used in 11 patients and

AZA (2 mg/kg/day) was used in 5 patients. All 17 patients

with hypertension were treated for their hypertension. Ten

patients with renal artery stenosis underwent surgery or

interventional radiographical stenting [29]. There are no

randomized therapeutic trials in TA. Immunomodulatory

drugs, such as MTX and mycophenolate mofetil, are

believed to be of some benefit in TA [6, 7, 52, 110–112].

The best observational evidence for treatment is for cor-

ticosteroids (to which 50% appear to respond) and MTX

(to which a further 50% appear to respond) [1].

In about one-fourth of the treated patients, remission is

never achieved [111]. Anti-TNF therapy may be a possible

beneficial agent for these patients. The initial series have

shown seemingly successful results with improvement in

patients with therapy-resistant TA. In an open-label trial of

anti-TNF therapy, including 15 patients with active, relap-

sing TA, addition of anti-TNF resulted in improvement in

14 of 15 patients [113].

The role of intravenous immunoglobulin, recombinant

IL-1 receptor antagonists, IL-4 and transforming growth

factor is speculative [113]. In patients with TA, platelet

and coagulation activities may be increased resulting

in a hypercoagulable state and sometimes in thrombus

formation. Therefore, some have advocated including

heparin or anti-platelet agents when treating PA [76, 82].

Percutaneous transluminal angioplasty (PCTA) is the

commonest palliative procedure performed, with a

success rate varying from 56 to 80% [7]. Stenosis in

TA can be effectively dilated by PCTA [114, 115].

However, despite providing short-term benefit, endovas-

cular revascularization procedures (bypass grafts, patch

angioplasty, endarterectomy, PTA, stent placement) are

associated with a high failure rate in patients with TA

[116–118].

Outcome

Anti-inflammatory therapy can lead to dramatic improve-

ment in TA. The 5-year survival rate in adults is as high as

94% [119]. The mortality rate in children, though, is as

high as 35% [4]. The outcome depends on the vessel in-

volvement and on the severity of hypertension [3]. In a

Turkish survey, one patient with pulmonary artery stenosis

died within the first 3 years and two patients underwent

nephrectomy [29]. In South Africa, the mortality rate was

22.5%; 7 of 31 patients died because of hypertension or

complications after kidney transplantation. One patient

died after EBV-associated haemophagocytic syndrome

[65]. In India, 2 of 24 patients (8.3%) died because of

renal failure and congestive heart failure [84]. Prevention

of organ damage may avoid worse outcome.

Summary

TA in children and adolescents is a potentially life-

threatening condition. The diagnosis should be suspected

in a child presenting with symptoms of hypertension,

fever, weight loss and vomiting. An awareness of TA,

and appropriate suspicion, is the first step for clinical

diagnosis. TA is thought to require early diagnosis for

the best outcome. Each child with suspected TA requires

a thorough diagnostic evaluation to exclude other condi-

tions on the differential diagnosis and to confirm the diag-

nosis of TA. Classical inflammatory markers have limited

sensitivity. Angiography is the standard for establishing

the diagnosis. Non-invasive methods such as ultrasound

may be considered, and may become more useful in

the future. Corticosteroids are the standard therapy.

Corticosteroids are frequently combined with other immu-

nomodulatory treatments especially for therapy-resistant

disease.

Rheumatology key messages

. In children with fever, vomiting and weight loss, TAshould be considered in the differential diagnosis.

. Musculoskeletal symptoms, cardiovascular signs,headaches, dyspnoea and abdominal pain maypoint to TA.

. The diagnosis of TA is supported by elevatedinflammatory markers. Suspected TA mandatesvascular imaging.

Disclosure statement: The authors have declared no

conflicts of interest.

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